Mimics of Sturge Weber syndrome

A 17 yo female came for CT Brain screening for seizures since last 5 years.

Non contrast CT study of Brain shows dense right parieto occipital gyriform calcification with an associated focal cortical Gliosis.

No other associated findings to support Sturge Weber Syndrome like ipsilateral hemiatrophy, compensatory thickening of ipsilateral hemicranium, Hyper pneumatisation of ipsilateral para nasal sinuses or mastoid, ipsilateral Choroid plexus enlargement. No ipsilateral port wine stain on face on clinical examination. 

DDs of Gyriform intracranial calcification mimicking the Sturge-Weber syndrome:

-Infarction.

-Purulent meningitis.

-Ossifying meningoencephalopathy and Meningitis Ossificans.

-Chemical meningitis secondary to subarachnoid fat from an Epidermoid tumor.

-Post encephalitis.

-Sequel of intrathecal administration of methotrexate for Leukemia.

-Skull irradiation for conditions like leukemia, lymphoma. 

-A consequence of cerebral folic acid deficiency in pt's with coeliac disease and epilepsy, the calcification appeared after treatment with anticonvulsants. Hypothesis is that the treatment contributing to the development of profound megaloblastic anemia and folic acid deficiency interfering with cerebral matabolism resulting in dystrohic calcification.

To see typical cases of Sturge Weber Syndrome Click here

Sturge Weber Syndrome CT Brain

Clinical details: A 14 y o male with mental retardation, seizures and a pink patch on left side fore head.

This non contrast CT brain shows:
Left hemiatrophy.
Dense left temporo parietal cortical gyriform calcification.
Poor development of ipsilateral hemicranium, thickening of bony calvarium.
Enlargement of ipsilateral choroid plexus.
Hyperpneumatisation of left frontal sinus.

Clinical and imaging findings are very typical of Sturge Weber syndrome.

Related posts:
Sturge-weber-syndrome Case 1
Sturge-weber-syndrome Case 3
Mimics-of-sturge-weber-syndrome

Sturge Weber syndrome Typical Imaging Findings

A 35 y o female brought unconscious with history of seizures since birth and mental retardation.

Clinically port wine stain on forehead, imaging finding on CT and MRI study of brain are very typical of Sturge Weber syndrome.

Non contrast CT Brain

MRI T2*GRE and T2w images

Typical imaging findings of SWS
Unilateral involvement.

Hemi cortical atrophy.

Dense tram track line gyriform calcification with leptomeningeal enhancement.

Ipsilateral choroid plexus enlargement.

Ipsilateral bony calvarial thickening, enlargement of para nasal sinuses and mastoid air cells seen in adults. 

Sturge Weber syndrome

SWS is an encephalo trigeminal angiomatosis, a rare congenital neurological and skin disorder, one of the phakomatoses. Unlike other neurocutaneous disorders (phakomatoses),  Sturge-Weber occurs sporadically without hereditary aetiology.

Characterised by port-wine stains of the face and ipsilateral lepto meningeal angioma.

Port-wine stain as birthmark on the forehead light pink to deep purple in colour, caused by abundance of capillaries around the ophthalmic branch of the trigeminal nerve.

Symptomatic with seizures from birth, mental retardation, glaucoma.

Related posts:
Mimics-of-sturge-weber-syndrome
Sturge-weber-syndrome Case 2
Sturge-weber-syndrome Case 3